内皮功能异常与肺动脉高压发病机制新认识

The Recent Understanding of Endothelial Dysfunction and the Pathogenic Mechanisms of Pulmonary Arterial Hypertension

肺动脉高压(PAH)是一组以肺血管阻力持续增加为特征的临床综合征,其发病机制至今尚未完全清楚。PAH的发生、发展涉及肺动脉内皮功能异常,一氧化氮、依前列醇、内皮素1等分泌失衡,炎症发生,内皮祖细胞参与血管重构,以及骨形成蛋白Ⅱ型受体基因突变等多种因素的交互作用。现从这几方面的新观点进行综述,相信这些研究将为更新的靶向疗法提供理论依据。

Pulmonary arterial hypertension （PAH） is a complex clinical syndrome which is clinically characterized by an increase in pulmonary vascular resistance. The pathogenic mechanisms of PHA remains unclear so far. Recent studies have proved that the development of PAH involves complex interactions of multiple factors, such as ： pulmonary artery endothelial dysfunction, secretion imbalance of nitric oxide, prostacyclin and endothelin-1, inflammation, endothelial progenitor cells involving vascular remodeling ; and the gene mutation of bone morphogenetic protein receptor-2. Here is to review the new ideas, which are belived to provide new theoretical basis for new targeted therapies.